Michaellis 10-year-old black male child who was admitted to the hospital because he was experiencing severe chest pain. He had been hospitalized on several previous occasions for vasoocclusive episodes that caused him to experience severe pain that could not be managed with nonprescription drugs such as ibuprofen. He was slightly jaundiced, short of breath and easily tired, and feverish. A chest x-ray was taken and was abnormal. An arterial blood sample showed a pO2 value of 6 kPa (normal is 10-13 kPa).
1. You suspect that Michael has sickle cell anemia and you have ordered an isoelectric focusing electrophoretic analysis of the chil’s lysed red blood cells. (Lysing the red blood cells releases the hemoglobin.) Draw a diagram of the predicted results. Why will this test allow you to diagnose this child’s disease?
2. In the emergency room, oxygen (100%) was administered to the patient. (Inspired air normally is about 20% oxygen.) Why was this an effective treatment?
3. You recall reading in the medical literature about a dramatic new drug treatment for sickle cell anemia, and you’d like to try it on this patient. The drug is hydroxyurea, and is thought to function by stimulating the afflicted person’s synthesis of fetal hemoglobin. Exactly how hydroxyurea stimulated fetal hemoglobin synthesis is unclear, but it is believed that hydroxyurea is metabolized to Nitric Oxide (NO), which binds to a soluble guanylate cyclase enzyme which catalyzes the synthesis of a second messenger, cyclic GMP (cGMP). The cGMP interacts with transcription factors in a manner that is not completely understood to induce the transcription of the fetal hemoglobin gene.
a. In a clinical study, patients who took hydroxyurea showed a 50% reduction in frequency of hospital admissions for severe pain, and there was also a decrease in the frequency of fever and abnormal chest x-rays. Why would increasing the synthesis of fetal hemoglobin result in alleviating the symptoms of sickle cell anemia?
b. Medical practioners who used hydroxyurea as a treatment for sickle cell anemia noted that their patients seemed to benefit from the administration of the drug long before the synthesis of fetal hemoglobin had time to take effect. It has recently been determined that hydroxyurea can react directly with the iron ion of oxy- and deoxyHb to form iron nitrosyl hemoglobin (HbNO). Why would this be of benefit to the sickle-cell anemic patient?
c. In aIDition, hydroxyurea has been shown to produce NO directly, in less than an hour after hydroxyurea administration. It’s also possible that HbNO could produce free NO, either directly or indirectly. NO is an mportant second messenger which, even in nanomolar amounts, stimulates vasodilation. How could production of NO help the sickle-cell anemic patient?
4.A year ago, at a conference, one of your colleagues told you that she had “cured” a patient of sickle cell anemia by performing a bone marrow transplant. Why would this procedure “cure” sickle cell anemia?
5. At the same conference, you attendedaa talk describing the discovery of a novel mode of action of NO: NO might be involved in oxygen delivery to the cells in a more direct manner-through reacting with hemoglobin, for example. This hypothesis was confirmed when the investigators showed that NO can react with hemoglobin to form S-nitrosohemoglobin. The NO reacts with the sulfhydryl group on Cys93 on the ? chain of hemoglobin to form the S-nitrosohemoglobin. The reaction is readily reversible, with SNO-Hb releasing NO under certain conditions.
a. A purified sample of hemoglobin is subjected to a chemical modification experiment in which an alkylating agent is aIDed to the hemoglobin. The alkylating agent Nethylmaleimide (NEM) reacts with sulfhydryl groups as shown in Fig. 1. The NEM has the ability to react with the Cys ?93 in oxy-Hb but not deoxy-Hb.
What does this tell you about the environment of Cys ?93?
Why Choose Us
Top quality papers
We always make sure that writers follow all your instructions precisely. You can choose your academic level: high school, college/university or professional, and we will assign a writer who has a respective degree.
Professional academic writers
We have hired a team of professional writers experienced in academic and business writing. Most of them are native speakers and PhD holders able to take care of any assignment you need help with.
If you feel that we missed something, send the order for a free revision. You will have 10 days to send the order for revision after you receive the final paper. You can either do it on your own after signing in to your personal account or by contacting our support.
All papers are always delivered on time. In case we need more time to master your paper, we may contact you regarding the deadline extension. In case you cannot provide us with more time, a 100% refund is guaranteed.
Original & confidential
We use several checkers to make sure that all papers you receive are plagiarism-free. Our editors carefully go through all in-text citations. We also promise full confidentiality in all our services.
24/7 Customer Support
Our support agents are available 24 hours a day 7 days a week and committed to providing you with the best customer experience. Get in touch whenever you need any assistance.
Try it now!
How it works?
Follow these simple steps to get your paper done
Place your order
Fill in the order form and provide all details of your assignment.
Proceed with the payment
Choose the payment system that suits you most.
Receive the final file
Once your paper is ready, we will email it to you.
No need to work on your paper at night. Sleep tight, we will cover your back. We offer all kinds of writing services.
You are welcome to choose your academic level and the type of your paper. Our academic experts will gladly help you with essays, case studies, research papers and other assignments.
Admission help & business writing
You can be positive that we will be here 24/7 to help you get accepted to the Master’s program at the TOP-universities or help you get a well-paid position.
Editing your paper
Our academic writers and editors will help you submit a well-structured and organized paper just on time. We will ensure that your final paper is of the highest quality and absolutely free of mistakes.
Revising your paper
Our academic writers and editors will help you with unlimited number of revisions in case you need any customization of your academic papers
Hello The Homework Helpers Support